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Objective: To determine the long-term psychological outcome of postlingually deafened adults who received multichannel cochlear implants and to relate the psychological outcome to audiological outcome., Design: Thirty-seven recipients of multichannel cochlear implants who participated in a prospective clinical trial completed psychological assessments before implantation and at regularly scheduled follow-ups through 54 mo of implant use. Standardized measures of affect, social function, and personality were used, and scores on these measures were correlated with asymptotic scores on several audiological measures., Results: Evidence of significant improvement on measures of loneliness, social anxiety, and distress were obtained within a year after implantation and throughout the duration of the follow-up period. For measures of assertiveness and marital satisfaction, improvement was apparent only after long-term implant use. Although favorable changes on the Minnesota Multiphasic Personality Inventory (MMPI) Depression Scale were evidenced only in the initial follow-up period, improvements on the MMPI Paranoia and Social Introversion Scales persisted throughout the 54 mo follow-up., Conclusion: Multichannel cochlear implant use is associated with long-term psychological benefit. Correlations between audiological outcome and psychological outcome, however, suggested that the relation between audiological benefit and psychological benefit is not simple.

The purpose of this study was to examine the appraisal and recognition of timbre (four different musical instruments) by recipients of Clarion cochlear implants (CIS strategy, 75- or 150-microsec pulse widths) and to compare their performance with that of normal-hearing listeners. Twenty-eight Clarion cochlear implant users and 41 normal-hearing listeners were asked to give a subjective assessment of the pleasantness of each instrument using a visual analog scale with anchors of "like very much" to "dislike very much," and to match each sound with a picture of the instrument they believed had produced it. No significant differences were found between the two different pulse widths for either appreciation or recognition; thus, data from the two pulse widths following 12 months of Clarion implant use were collapsed for further analyses. Significant differences in appraisal were found between normal-hearing listeners and implant recipients for two of the four instruments sampled. Normal-hearing adults were able to recognize all of the instruments with significantly greater accuracy than implant recipients. Performance on timbre perception tasks was correlated with speech perception and cognitive tasks.

Objective: To examine whether children perform better when they receive cochlear implants when they are 2 to 4 years of age than when they are older, and to determine whether 4-year performance can be predicted from 1-year results., Method: Children in two age groups (2 to 4, 4 to 9 years) were tested for performance, and the age groups were compared. Children were also tested 1 and 4 years after implantation., Results: The results suggest that the "implanted young" group scored higher than the "implanted old" group after 36 months, and that 1-year performance is helpful in predicting 4-year performance., Conclusion: It may be desirable for children to undergo implantation when they are under 2 years of age, provided that appropriate selection criteria can be determined.

In this investigation we measured the performance of 50 prelingually deaf children on several speech perception tests. Children were from 2 to 15 years of age, and some children were tested with as much as 5 years of cochlear implant use. Speech perception tests included the recognition of stress pattern, consonants, vowels, words, and sentences. The audiovisual perception of consonants was also measured. Average results indicated that gains were being made in the perception of stress and words in a closed-set context within 1 year from implantation. The perception of words in an open-set context demonstrated much slower increases over time. Large individual differences were observed. Some preliminary data suggest that children who receive implants before the age of 4 years obtain higher scores, on average, than children who receive implants after the age of 5 years. Some children become part-time users or nonusers of their cochlear implants. The average results from 18 congenitally deaf children were significantly higher than the average results from 12 children with prelingually acquired deafness after 3 years of implant use. Information on vowel and consonant features shows increases in performance after 2 years of cochlear implant use, with the exception of the place feature. For this feature, no changes were observed. Vision-alone testing indicated that lipreading performance increased over time. An audiovisual enhancement provided by the cochlear implant was observed for all features.

This study examined the average and individual performance over time of 49 adult cochlear implant subjects. Subjects were randomly assigned to receive either the Ineraid cochlear implant, with analog processing, or the Nucleus cochlear implant, with feature-extraction processing. All subjects had postlingual profound bilateral sensorineural hearing loss and received no significant benefit from hearing aids before implantation. Group data were examined in two ways. First, only subjects who had complete data over the test period were examined. Second, an analysis of all available data was carried out by mixed linear-model analysis. In this analysis, to account for missed follow-ups at the planned intervals, data consisting of the observations closest in time to the planned test times were modeled by natural splines with knots at the planned follow-up times. Contrasts between all pairs of planned follow-up times for each device were tested, as were contrasts between devices at each planned follow-up time. Results indicated little difference between the performance of the Ineraid and Nucleus subjects in their level of performance or their rate of learning. Postimplantation performance was typically superior to preimplantation performance within 9 months, and continued to improve up to 18-30 months depending on the speech perception measure. In some subjects, improvements in speech perception measures were observed up to four or five years postimplantation. There was also evidence that three subjects had a decrement in overall speech perception performance, although their postimplantation scores were always higher than their preimplantation scores. In at least one subjects this was likely a result of age-related cognition decrements.

Objective: This study compares the musical perception of 17 adult recipients of the Nucleus cochlear implant using two different formant extraction processing strategies (F0F1F2 and MPEAK)., Design: Over a 12 mo period, participants were alternately switched between two strategies every 3 mo. Performance was evaluated using three measures of rhythmic and sequential pitch perception., Results: Three individuals performed significantly better with the MPEAK strategy on one particular rhythm task, 11 participants performed better with the MPEAK strategy on another rhythm task, and no significant differences were found between the two strategies on a sequential pitch pattern task., Conclusions: Neither strategy seems clearly superior for perception of either sequential pitch or rhythmic patterns.

This study focused on long-term speech perception performances of 34 prelingually deafened children who received multichannel cochlear implants manufactured by Cochlear Corporation. The children were grouped by the age at which they received cochlear implants and were characterized by the amount of time they used their device per day. A variety of speech perception tests were administered to the children at annual intervals following the connection of the external implant hardware. No significant differences in performance are evident for children implanted before age 5 compared to children implanted after age 5 on closed-set tests of speech perception ability. All children demonstrated an improvement in performance compared to the pre-operative condition. Open-set word recognition performance is significantly better for children implanted before age 5 compared to children implanted after age 5 at the 36-month test interval and the 48-month test interval. User status, defined by the amount of daily use of the implant, significantly affects all measures of speech perception performance except pattern perception.

Twenty children who have worn a Cochlear Corporation cochlear implant for an average of 33.6 months participated in a device-on/off experiment. They spoke 14 monosyllabic words three times each after having not worn their cochlear implant speech processors for several hours. They then spoke the same speech sample again with their cochlear implants turned on. The utterances were phonetically transcribed by speech-language pathologists. On average, no difference between speaking conditions on indices of vowel height, vowel place, initial consonant place, initial consonant voicing, or final consonant voicing was found. Comparisons based on a narrow transcription of the speech samples revealed no difference between the two speaking conditions. Children who were more intelligible were no more likely to show a degradation in their speech production in the device-off condition than children who were less intelligible. In the device-on condition, children sometimes nasalized their vowels and inappropriately aspirated their consonants. Their tendency to nasalize vowels and aspirate initial consonants might reflect an attempt to increase proprioceptive feedback, which would provide them with a greater awareness of their speaking behavior.

This study compares the Nucleus F0F1F2 and F0F1F2B3B4B5 (also known as "Multipeak") of "Mpeak") processing schemes in 17 patients wearing the Mini Speech Processor. All patients had at least 18 months implant experience using the F0F1F2 processing strategy. For this study, they were switched to the F0F1F2B3B4B5 processing strategy for 3 months. They then returned to using the F0F1F2 strategy for 3 months, then used the F0F1F2B3B4B5 strategy again for 3 months, and lastly used the F0F1F2 strategy for 3 months. Performance' was evaluated with both schemes after each interval, using speech recognition tests and subjective ratings. Overall, differences between the results for the two processing schemes were not large. Average performance was somewhat better for the F0F1F2B3B4B5 strategy for word and sentence identification, but not for any of the other speech measures. Superior performance was observed in 8 patients with the F0F1F2B3B4B5 strategy. However, 6 of the 8 individuals were significantly better on only one of the six speech measures in the test battery. The other 2 patients performed better on two of the speech measures. Superior performance was also observed in 3 patients with F0F1F2 strategy for consonant recognition. For the remaining patients, there was little difference in their performance with the two strategies. Information transmission analyses indicated that the F0F1F2B3B4B5 strategy transmitted consonant duration and frication cues more efficiently than F0F1F2. Experience with one strategy appeared to benefit performance with the other strategy.

A clinical trial of three different multichannel cochlear implants is in progress at The University of Iowa. Fifty postlingually deaf adults participated in a prospective randomized study of the Nucleus implant and the Ineraid device. Subsequently, 23 consecutive postlingually deaf adults have been implanted with the Clarion system. The speech perception performance obtained after 9 months of implant use with all devices was compared. In addition, Clarion patient results at 9 months were compared with long-term (36 months) results from subjects using the other two multichannel implants. Preliminary speech perception scores on the Iowa sentence test (sound-only) and NU-6 words (sound-only) demonstrate that the Clarion subjects, as a group, perform at higher levels than subjects using the other two systems. A wide range of performance is observed with all devices. The continuous interleaved pulsatile strategy of the Clarion device appears to be advantageous for this group of subjects.

Forty-two postlingually deafened adult patients, 21 with a formant extraction version of the Nucleus cochlear implant and 21 with the Ineraid cochlear implant (analog processing), were evaluated on a series of speech perception tests after using their implants for about 3.5 years. A wide range of performance was observed across patients for both devices. All but 4 patients showed an enhancement in their lipreading ability with the implant. Word recognition averaged about 14-19% correct, and word recognition in sentences averaged about 43-49% correct for the two implant groups. Average performance was superior with the Ineraid implant on consonant recognition in noise. An information transmission analysis suggested that vowel perception was influenced by first- and third-formant frequency for the Nucleus, and first-formant and fundamental frequency for the Ineraid patients. It appeared that the Ineraid device was more effective, on average, at conveying information about consonant nasality and frication. For consonant perception, nasality and frication contributed most to the total information transmitted for both implant types. Both devices had difficulty conveying information about vowel second-formant frequency and consonant place information. These scores at 3.5 years are substantially elevated from preoperative performance and, overall, the patients clearly benefit from their implant.

The four purposes of this investigation were to assess whether children acquire intelligible speech following prolonged cochlear-implant experience and examine their speech error patterns, to examine how age at implantation influences speech acquisition, to assess how speech production and speech perception skills relate, and to determine whether cochlear implant recipients who formerly used simultaneous communication (speech and manually coded English) begin to use speech without sign to communicate. Twenty-eight prelinguistically deafened children who use a Nucleus cochlear implant were assigned to one of three age groups, according to age at implantation: 2-5 yrs (N = 12), 5-8 yrs (N = 9), and 8-15 yrs (N = 7). All subjects had worm a cochlear implant for at least 24 mos, and an average of 36 mos. All subjects used simultaneous communication at the time of implantation. Subjects performed both imitative and structured spontaneous sampling speech tasks. The results permit the following conclusions: (a) children who have used a cochlear implant for at least 2 yrs acquire some intelligible speech; (b) children who receive a cochlear implant before the age of 5 yrs appear to show greater benefit in their speech production skills than children who are older, at least after a minimum of 2 yrs of use; (c) children who recognize more speech while wearing their cochlear implants are likely to speak more intelligibly; and, (d) signing does not disappear from a child's communication mode following implantation.

Postlingually deafened children, using multichannel cochlear implants, have achieved substantial improvement in their speech perception abilities and, in many instances, the results are better than in postlingually deafened adults. It has been suggested that children with prelingually acquired and congenital deafness would not receive similar benefits, since they have not developed an auditory memory. The purpose of this study is to analyze the speech perception and production performance over time of prelingually deafened children who have been using a multichannel cochlear implant for 1-5 years. Preliminary results comparing the effects of age at implantation and etiology of deafness on performance are also examined.

To predict the audiological outcomes of 2 multichannel cochlear implants, a preoperative battery of historical, audiological, electrophysiologic, and psychologic variables from 48 postlingually deafened adults was tested in a prospective randomized clinical trial. Multivariate analyses were used to select and combine these preoperative variables in a predictive index that was significantly related to audiological outcome at 9 months. The preoperative variables included in the predictive index were duration of profound deafness, speech reading ability, residual hearing, cognitive ability, measures of compliance and engagement with treatment, and use of nonverbal communication strategies. The preoperative predictive index had correlations of .81 with the Iowa Sentences Test, and .78 with the NU-6 word understanding scores, both obtained in a sound-only test. Probability and percentile curves generated from these data offer considerable optimism in forecasting the range of likely audiological outcomes that would be realized by postlingually deafened adult candidates for multichannel cochlear implants.

Consecutive recipients of multichannel cochlear implants participated in preimplant as well as 9-month and 18-month psychological evaluations. Before receiving a cochlear implant, psychological tests indicated that the implant recipients were more depressed, suspicious, socially isolated, lonely, and socially anxious than was the general population. After 18 months of implant use, there was a significant reduction in depression, loneliness, social anxiety, social isolation, and suspiciousness. These changes in psychological state did not correlate with improved performance on audiological measures. The data suggest that although cochlear implants can have a positive effect on the emotional and behavioral status of persons with acquired postlingual profound deafness, the psychological outcome of implants is not simply a function of the audiological benefit assessed with standardized speech-based audiological tests.

The audiologic performance of 54 postlingually deafened adults wearing cochlear implants was uniformly evaluated. The participants had 9 months' or more experience with one of five different cochlear prostheses (Los Angeles Single Channel (N = 11), Vienna Single Channel (N = 4), Melbourne Multichannel (N = 18), Utah Multichannel (N = 19), San Francisco Multichannel (N = 2). The multichannel designs enabled participants to recognize more environmental sounds, provided more speech reading enhancement, and enabled most users to understand limited speech in the sound-only condition, compared to the single-channel implant group.

Hearing talkers produce shorter vowel and word durations in multisyllabic contexts than in monosyllabic contexts. This investigation determined whether a similar effect occurs for deaf talkers, a population often characterized as lacking coarticulation in their speech. Four prelingually deafened adults and two hearing controls produced three sets of word sequences. Each set included a kernel word and six derived forms (e.g., "speed," "speedy," "speeding," etc.). The derived forms were created by adding unstressed and stressed syllables to the kernel form. A spectrographic analysis indicated that the deaf subjects did not always decrease word and vowel durations for the derivatives. Unlike hearing speakers, they often did not reduce vowel segments more than consonant segments. Three explanations are forwarded for the shortening effects. One relates to the implementation of temporal rules, the second concerns the organization imposed upon the articulators to produce speech, and the third suggests a language-independent vocal tract characteristic. The role of auditory information in developing the shortening effects is also considered.

Context: Mutations in the GJB2 gene are the most common known cause of inherited congenital severe-to-profound deafness. The carrier frequency of these mutations is not known.Objectives: To determine the carrier rate of deafness-causing mutations in GJB2 in the midwestern United States and the prevalence of these mutations in persons with congenital sensorineural hearing loss ranging in severity from moderate to profound, and to derive revised data for counseling purposes.Design: Laboratory analysis, performed in 1998, of samples from probands with hearing loss for mutations in GJB2 using an allele-specific polymerase chain reaction assay, single-strand conformation polymorphism analysis, and direct sequencing.Setting and Subjects: Fifty-two subjects younger than 19 years sequentially referred to a midwestern tertiary referral center for hearing loss or cochlear implantation, with moderate-to-profound congenital hearing loss of unknown cause, parental nonconsanguinity, and nonsyndromic deafness with hearing loss limited to a single generation; 560 control neonates were screened for the 35delG mutation.Main Outcome Measure: Prevalence of mutations in the GJB2 gene by congenital deafness status.Results: Of 52 sequential probands referred for congenital sensorineural hearing loss, 22 (42%) were found to have GJB2 mutations. The 35delG mutation was identified in 29 of the 41 mutant alleles. Of probands' sibs, all homozygotes and compound heterozygotes had deafness. Fourteen of 560 controls were 35delG heterozygotes, for a carrier rate expressed as a mean (SE) of 2.5% (0.66%). The carrier rate for all recessive deafness-causing GJB2 mutations was determined to be 3.01% (probable range, 2.54%-3.56%). Calculated sensitivity and specificity for a screening test based on 35delG mutation alone were 96.9% and 97.4%, respectively, and observed values were 94% and 97%, respectively.Conclusions: Our data suggest that mutations in GJB2 are the leading cause of moderate-to-profound congenital inherited deafness in the midwestern United States. Screening of the GJB2 mutation can be offered to individuals with congenital deafness with high sensitivity and specificity by screening only for the 35delG mutation. A positive finding should establish an etiologic diagnosis and affect genetic counseling. [ABSTRACT FROM AUTHOR]